We present the case of a 3-year-old girl with acute lymphoblastic leukemia who developed isolated central nervous system relapse while receiving chemotherapy 10 months after diagnosis. The child achieved a second remission on retreatment with systemic and intrathecal chemotherapy. She then underwent myeloablative chemotherapy and radiation therapy followed by infusion of her own umbilical cord blood, which the parents had saved after her delivery. She is now doing well and is in complete remission 20 months after cord blood transplantation. In this first report of autologous cord blood transplantation for treatment of childhood leukemia, we discuss the safety and feasibility of this procedure as well as some of the uncertainties surrounding autologous cord blood collection and usage.

We report a female patient with Seckel syndrome who developed acute myeloid leukaemia at the age of 26 years. Analysis of bone marrow chromosomes showed an abnormal clone with abnormalities involving multiple chromosomes, including monosomy 7, trisomy 8, trisomy 11, and loss of the long arm of chromosome 5. After treatment with chemotherapy, the patient experienced severe toxicity with profound bone marrow aplasia and died of pneumonia two months later. We suggest that patients with Seckel syndrome may be at risk of developing myelodysplasia and acute myeloid leukaemia. They may also have poor tolerance to cytotoxic therapy.

Forty-seven children with nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) were treated by the Hematology-Oncology Service at Texas Children's Hospital, Houston, Texas, between 1958 and 1990. The male:female ratio was 1:1, and the median age was 11 years (3 weeks-16 years). A preexisting condition was found in 9/47 (19%) patients including neurofibromatosis (3), Down's syndrome (1), spina bifida (1), congenital facial asymmetry (1), giant pigmented nevus (1), juvenile onset diabetes mellitus (1), and acquired immune deficiency syndrome (1). The site of primary tumor was head and neck (3), trunk (33), and extremities (11). Twenty-four patients (51%) have survived free of disease with a median follow-up of 5 years (4 months-22 years). No patient whose disease recurred achieved a second remission. Of the 19 patients with group I disease, 16 (84%) survived free of disease. Wide excision of the primary tumor, with no microscopic residual disease, was associated with the greatest chance of disease-free survival.

We report a case of a child with precursor-B acute lymphoblastic leukemia (ALL) who experienced refractory thrombocytopenia and massive splenomegaly during standard induction chemotherapy. He was diagnosed with hemophatocytic lymphohistiocytosis (HLH) during induction. Clinical and laboratory evaluation showed no evidence of infectious cause to HLH. Pancytopenia and HLH persisted after consolidation therapy even with remission from leukemia. After failure to control HLH with ALL-directed therapy and HLH-directed therapy, the patient underwent unrelated donor hematopoietic stem cell transplantation 8 months after diagnosis. He is 34 months post-transplant and in remission from leukemia and HLH.

No abstract

A case of pyoderma gangrenosum (PG) in a 14-year-old boy with acute myelogenous leukemia (AML) is described. The onset of pyoderma gangrenosum coincided with the relapse of AML. The lesions responded dramatically to treatment with oral prednisone despite the persistence of leukemia. Pyoderma gangrenosum should be included in the differential diagnosis of any nodular, pustular, or necrotic cutaneous eruption in children with leukemia.

Children who receive head, neck, or chest radiotherapy for various primary malignancies have increased risk for secondary thyroid malignancy. Thyroid nodules are difficult to identify by physical examination and/or laboratory tests. Thyroid ultrasound can detect non-palpable nodules without adverse side effects. We performed a retrospective chart review of 36 patients who received radiotherapy and underwent thyroid ultrasound. Forty-seven percent (n = 17) had ≥1 nodule(s) detected. Seven patients underwent thyroidectomy; four of whom were diagnosed with thyroid malignancy. Our study suggests routine use of thyroid ultrasound in high-risk patients detects subclinical thyroid nodules and potential thyroid malignancy post-radiotherapy.

No abstract